8% of a series of 61 patients with RDEB with a mean age of confirmation of diagnosis of 8.7 years99. Osteoporosis and osteopenia: A study of 39 children indicated that patients with RDEB and JEB had lower bone mineral density scores than control children56. In this study, a correlation was noted between low bone mass and reduced body mobility. 7.3.3 Management.  A systematic review of randomized controlled trials of treatments

for inherited forms of EB was published in 2008100. Up to the 1 April 2007, the researchers identified five randomized double-blind placebo-controlled crossover trials. None of the studies showed a benefit of the intervention over placebo100. There is still no reliable trial evidence for interventions in inherited EB. Gene, protein, and cell therapies are being researched, but until reliable evidence becomes available, most treatment of EB is directed towards preventative, supportive, Tamoxifen symptomatic, and palliative goals. Prevention of blisters:  Protection of the fragile skin of EB is of utmost importance (Images 37–38). A cool environment and skin lubrication can help lessen blister formation. Sheepskin is used for padding car seats, infant seats, and other surfaces. Young children should not been picked up under the arms, but be lifted from the bottom and the back of the neck. Clothing

should be made of soft fabric and simple design26. Management of EB wounds:  Most EB wound care techniques consist of multiple layers of bandages or sterile nonadherent ICG-001 manufacturer materials (Images 38–40). Dressings are changed on a daily basis or every second day. Blisters must be drained, ideally under sterile conditions, to prevent them enlarging and giving rise to larger erosions33. Dressings should aim to maintain appropriate moisture, be nonadherent, atraumatic, promote a healthy wound bed, reduce pain, and increase speed of re-epithelialization.

(Image 41) Surgical interventions:  Patients with EB, especially RDEB, often require surgery within the oral cavity, gastrointestinal tract, and on the hands. Among the challenges for anaesthesiologists are microstomia, ankyloglossia, intraoral blistering, and sloughing, and the possible need for tracheostomy. When procedures Leukotriene-A4 hydrolase under general anaesthesia are planned, it is best to coordinate as many interventions as possible to avoid repeated anaesthesia26. Anaesthetic managementC:  Anaesthetic management of patients with EB presents several difficulties as a result of mucosal fragility, severe scarring of all tissues, and oesophageal strictures increasing the risk of regurgitation and aspiration during anaesthesia. Coordinated care with dermatologists, surgeons, and nurses is essential for anaesthesia and perioperative management in patients with RDEB (Table 2).57 Nonsurgical interventions– It is a common practice to mechanically separate the digits with gauze wraps on a daily basis in an attempt to prevent, minimize, or delay the EB-associated pseudosyndactyly.